Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001917908 | SCV002170719 | pathogenic | Jeune thoracic dystrophy; Nephronophthisis | 2024-05-26 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg858*) in the TTC21B gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in TTC21B are known to be pathogenic (PMID: 18327258, 21068128, 21258341, 23559409, 24876116, 25492405, 27491411, 29068549). This variant is present in population databases (no rsID available, gnomAD 0.002%). This premature translational stop signal has been observed in individual(s) with nephronophthisis-related ciliopathy (PMID: 27491411). ClinVar contains an entry for this variant (Variation ID: 1399888). For these reasons, this variant has been classified as Pathogenic. |